This protocol addresses the next treatment step for patients with paraneoplastic cerebellar degeneration in whom initial immunotherapy — intravenous immunoglobulins (IVIG) or plasma exchange (PLEX), combined with corticosteroids — has not achieved adequate reduction of circulating autoantibodies.
The preceding approach used IVIG or plasma exchange (PLEX), either added simultaneously to corticosteroids in patients with severe symptoms or rapid clinical worsening, or started as second-line therapy when corticosteroid monotherapy showed insufficient effect. Escalation is indicated when the goal of reducing circulating autoantibodies has not been met.
This protocol defines a structured maintenance immunotherapy strategy — one that targets specific immune cell populations — for patients after the above failure condition. The complete regimen, including the choice between agents, applicable alternatives, and the conditions under which each applies, is available in the full protocol.
DOI: 10.3390/brainsci11111414
According to a recent survey, most therapists chose rituximab over cyclophosphamide in a setting with a supposed and unknown antibody.
Strategies targeting all types of immune cells (mainly T- and B-cells) include substances such as azathioprine, mycophenolate mofetil, and cyclophosphamide.
In case of intended depletion of CD20 positive plasma cells, rituximab is a well-tolerated substance.
Especially after good clinical response by PLEX, even in the absence of a specific antibody, this is a feasible therapeutical approach.
In case of contraindications or adverse effects under therapy with immunosuppressants, monthly administrations of IVIG or performance of PLEX can be considered in the presence of good clinical response.
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